Primary systemic sclerosis heart involvement: A systematic literature review and preliminary data-driven, consensus-based WSF/HFA definition.

Primary heart involvement in systemic sclerosis may cause morpho-functional and electrical cardiac abnormalities and is a common cause of death. The absence of a clear definition of primary heart involvement in systemic sclerosis limits our understanding and ability to focus on clinical research. We aimed to create an expert consensus definition for primary heart involvement in systemic sclerosis. A systematic literature review of cardiac involvement and manifestations in systemic sclerosis was conducted to inform an international and multi-disciplinary task force. In addition, the nominal group technique was used to derive a definition that was then subject to voting. A total of 16 clinical cases were evaluated to test face validity, feasibility, reliability and criterion validity of the newly created definition. In total, 171 publications met eligibility criteria. Using the nominal group technique, experts added their opinion, provided statements to consider and ranked them to create the consensus definition, which received 100% agreement on face validity. A median 60(5-300) seconds was taken for the feasibility on a single case. Inter-rater agreement was moderate (mKappa (95% CI) = 0.56 (0.46-1.00) for the first round and 0.55 (0.44-1.00) for the second round) and intra-rater agreement was good (mKappa (95% CI) = 0.77 (0.47-1.00)). Criterion validity showed a 78 (73-84)% correctness versus gold standard. A preliminary primary heart involvement in systemic sclerosis consensus-based definition was created and partially validated, for use in future clinical research.

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Declaration of conflicting interests: The author(s) declared the following potential conflicts of interest with respect to research, authorship and/or publication of this article: C.B. reports consultancy fee from Actelion, Eli Lilly; grants from Gruppo Italiano Lotta alla Sclerodermia (GILS), Fondazione Italiana Ricerca sull’Artrite (FIRA), European Scleroderma Trial and D.E.F. reports grant/research support from Corbus, Galapagos GSK, Pfizer, Talaris, CSL Behring, Mitsubishi; Consultant fees from Actelion, Amgen, Corbus, Galapagos, Novartis, Pfizer, Roche/Genentech, Talaris, CSL Behring, Boehringer Ingelheim. G.D.L. received honoraria from SOBI, Novartis, Pfizer, MSD, Celgene. L.G. has received consultancy fees from GE Healthcare outside the submitted work. Y.A. reports personal fees from Actelion, Bayer, BMS, Boehringer and Curzion, and grants and personal fees from Inventiva, Roche, and Sanofi. M.K. has received consultancy fees and/or research grant funding from Abbvie, Actelion Pharmaceuticals, Astellas, Bayer, Boehringer Ingelheim, Chugai, Corbus, CSL Behring, Eisai, Mochida, Nippon Shinyaku, Novartis, Ono, Pfizer, Reata, and Tanabe-Mitsubishi. C.P.D. has received consultancy fees and/or research grant funding from Actelion, GlaxoSmithKline, Bayer, Sanofi-Aventis, Inventiva, Boehringer Ingelheim, Roche, CSL Behring, UCB Pharma, Leadiant Biosciences, Corbus, Acceleron. D.K. reports personal fees from: Actelion, Abbvie, Bayer, Boehringer-Ingelheim, Chemomab, Corbus, CSL Behring, Genentech/Roche, Gilead, GSK, Mitsubishi Tanabi, Sanofi-Aventis, UCB Pharma. He reports grants from Bayer, Boehringer-Ingelheim, Genentech/Roche, Pfizer, Sanofi-Aventis and has stock options in Eicos Sciences, Inc. T.K. reports consultancy fee and grant funding from Actelion. E.Z. reports consultancy fee from GlaxoSmithKline. M.M.-C. reports grant and personal fees from Actelion, personal fees from Biogen, personal fees from Bayer, personal fees from Boehringer Ingelheim, personal fees from CSL Behring, personal fees from Eli-Lilly, outside the submitted work. M.H.B., A.D., R.B.D., A.G., M.P., Y.A.S., K.B., A.S., I.M., A.B., G.H., A.X., Y.I., G.M.-M., L.T., S.M., A.L.P.C., C.T., A.R., K.K., S.P., E.R.B., R.M., I.G., F.T., P.S.: no conflicts of interest to declare.